Relapsing polychondritis is a rare and multi-system autoimmune disease of unknown etiology characterized by inflammation and destruction of cartilaginous structures. Its clinical manifestations include recurrent chondritis of the ears, nose, pinna, peripheral joints, and laryngotracheobronchial tree and can be life-threatening in advanced cases of laryngotracheal stenosis. Because of the rarity of relapsing polychondritis and lack of understanding of its pathogenesis, there is no standard medical therapy, and treatment is tailored according to disease activity and site of organ involvement. Read More

Authors:

Clinic of Rheumatology and Internal Medicine, Wrocław University Hospital, Poland.

Objectives: Relapsing polychondritis (RP) is a rare disease characterised by recurrent inflammation of the cartilaginous structures and proteoglycan-rich organs. The aim of this case series study is to share the 10-year clinical experience of our department in diagnosing RP patients in the context of data from available published studies.

Material And Methods: A retrospective case analysis of 10 patients with symptoms of RP, hospitalised at the Department of Rheumatology and Internal Diseases of Wrocław University Hospital between January 2008 and December 2018. Read More

Authors:

Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine.

Objectives: Relapsing polychondritis (RP) is a very rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We examined the clinical characteristics, management, and outcomes of Japanese RP patients.

Methods: We identified 8 RP cases in our department between 2003 and 2017. Read More

Recurrentpolyhedritis is a rare rheumatic disorder with a wave-like course, presumably ofautoimmune nature,characterizedbya widespread inflammatorylesion of the cartilaginous structures and other connective tissue of the ears, joints, nose, larynx, trachea, eyes, valvular heart, kidneys and blood vessels.The description of the own observation of recurrent polychondritis. Read More

Background And Objectives: An auricular pseudocyst is not uncommon in routine ENT clinical practice, it occurs when fluid accumulates between the intracartilaginous spaces of the auricle. Many treatment modalities have been proposed such as incision and drainage of the cyst, simple needle aspiration, tight bandaging with dental rolls, however recurrence and cosmetic problems are still noted in some cases. The aim of this article was to discuss our experience of surgical treatment of intractable auricular pseudocysts with marsupialisation, deroofing and anterior cartilage leaflet removal along with compression suture therapy. Read More

Objectives: Relapsing polychondritis is a rare, multi-systemic and inflammatory condition of unknown origin. We currently lack a core set of measures to assess and follow damage in patients suffering from this condition. Our primary aim was to derive a disease-specific damage measuring tool for relapsing polychondritis, the Relapsing Polychondritis Damage Index (RPDAM). Read More

Authors:

Relapsing polychondritis (RP) is a rare autoimmune disorder affecting cartilage. Limbic encephalitis is a rare central nervous system manifestation of RP. This current case report describes a 66-year-old Chinese male patient who complained of developing myoclonus in the left leg, ataxia and speech difficulties 3 weeks prior to hospital admission. Read More

Authors:

Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK.

A 65-year-old man presented with transient neurological symptoms, followed by rapid cognitive decline, myoclonus and fevers. He had evidence of scleritis and an arthropathy. This paper reports the clinicopathological conference discussed at the Association of British Neurologists Annual Meeting 2017. Read More

Authors:

Clinical Assistant Professor, University of Illinois College of Medicine at Peoria, Peoria, IL, USA.

Meningoencephalitis is a rare complication of relapsing polychondritis. We report a case of a 25-year-old male who presented with visual hallucinations and symptoms of depression and anxiety, white matter changes on MRI, and CSF lymphocytosis, along with inflammatory chondritis seen in his auricle cartilage biopsy. Eventually he was given the diagnosis of RP presenting with meningoencephalitis based on CSF analysis, brain MRI findings, negative serologies, and neurologic exam findings. Read More

Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). Read More

Authors:

Objective: To analyze the characteristics of clinical manifestation, imaging, fiber-bronchoscope finding and pulmonary function testing in the patients of relapsing polychodriti with respiratory symptoms.

Methods: The clinical data of 33 patients with RP in West China Hospital of Sichuan University from May 2007 to May 2017 were analyzed retrospectively. The patients were divided into respiratory group (21 cases) and non-respiratory group (12 cases) based on whether the respiratory symptoms were main involvement, and the data of the two groups were statistically analyzed. Read More

The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. Read More

Purpose: Idiopathic uveitis is frequently the most common diagnosis in series from uveitis clinics. This study sought to determine the percentage of patients initially diagnosed as idiopathic, noninfectious uveitis referred to a tertiary uveitis center who were subsequently found to have an identifiable cause of uveitis.

Authors:

Department of Immunology and Medicine, Institute of Medical Science, and Division of Rheumatology and Allergology, St. Marianna University School of Medicine, Kawasaki, Japan.

Relapsing polychondritis (RP) is a multisystem disorder of cartilaginous tissues. Previously, we found that patients with respiratory involvement and patients with auricular involvement were mutually exclusive in the RP cohort, which suggests a strong inverse relationship between respiratory and auricular involvement. Here, we examined the clinical manifestation patterns in a subgroup of patients with respiratory involvement (R subgroup) and a subgroup of patients with auricular involvement (A subgroup) and investigated the clinical and laboratory characteristics of each subgroup. Read More

Authors:

Department of Otorhinolaryngology, "Iuliu Haţieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania;

Giant cell arteritis (GCA) is a large-vessel vasculitis with rarely described respiratory initial manifestations. We report such a case presenting with hoarseness, stridor, cough and dyspnea, in which a tracheomegaly with tracheomalacia was found. No signs of relapsing polychondritis were present. Read More

Authors:

Department of Histology and Embryology, Medical University of Warsaw, Poland.

The purpose of this work was to establish, whether rat chondrocyte associated antigen, transmembrane Tmp21 protein belonging to the p24 protein family may immunize rats and thus be included into the panel of immunogens potentially involved in cartilage pathology. For immunization of rats extract from cultured chondrocytes containing surface chondrocyte proteins suspended in incomplete Freund's adjuvant was used. Control animals were injected with incomplete Freund's adjuvant without chondrocyte extract. Read More

Observational study of patients with relapsing polychondritis (RPC) and brief evaluation of widely used diagnostic criteria. A retrospective analysis of 18 patients with RPC treated in the past 15 years at the Charté-Universitätsmedizin Berlin was performed. Three different diagnostic criteria were applied to our cohort. Read More

Authors:

Department of Clinical and Experimental Medicine, Section of Dermatology, University of Messina, 98125 Messina, Italy.

Relapsing polychondritis is an immune-mediated systemic disease characterized by recurrent episodes of inflammation of cartilaginous and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment of the involved structures. Auricular and nasal chondritis and/or polyarthritis represent the most common clinical features, but potentially all types of cartilage may be involved. Because of the pleomorphic nature of the disease, with non-specific symptoms at the onset, the diagnosis of relapsing polychondritis is often delayed. Read More

Authors:

Relapsing polychondritis (RP) is an autoimmune disorder that often occurs concomitantly with other autoimmune diseases, though RP has been infrequently associated with ankylosing spondylitis (AS). There is a small, but growing, body of the literature demonstrating case reports describing RP secondary to AS in patients treated with tumor necrosis alpha inhibitors (TNFi's). We present the first case in which RP developed in AS while treated with an interleukin 17A inhibitor (IL-17Ai), secukinumab. Read More

Authors:

Purpose: Relapsing polychondritis is a rare multiorgan disease characterized by repeated episodes of inflammation and deterioration of cartilages. We report a case of relapsing polychondritis that presented with severe panuveitis.

Observations: A 53-year-old man visited our hospital because of ocular pain in both eyes of 2 weeks' duration. Read More

Authors:

Department of Dermatology, University of California-San Diego School of Medicine, San Diego, California, USA. Electronic address:

The cutaneous manifestations of the common rheumatologic disorders, such as systemic lupus erythematosus, dermatomyositis, and systemic sclerosis, are well known. In contrast, the dermatologic findings of less common rheumatologic disorders, including Sjögren syndrome, mixed connective tissue disease, and relapsing polychondritis, are less widely known. The cutaneous manifestations of these connective tissue disorders are reviewed. Read More

Introduction We present a single surgeon's experience of open augmentation rhinoplasty with autogenous L-shaped costal cartilage grafts, with long-term patient-reported outcome data. We highlight the salient operative steps and outline the peri-operative care required to optimise outcomes. Materials and Methods A retrospective review of eleven such augmentation rhinoplasties performed between 2008 and 2016 was undertaken. Read More

Authors:

Department of Gastroenterology, Medical School of Ioannina, Ioannina, Greece.

Inflammatory bowel disease (IBD) is a multisystemic disease. The ear is a rare but recognized site of extraintestinal manifestations of IBD. In external ear, the more common manifestations of IBD are pyoderma gangrenosum, metastatic Crohn's disease and relapsing polychondritis and the treatment includes corticosteroids and anti-TNF agents. Read More

Hyaline cartilages, fibrocartilages and elastic cartilages play multiple roles in the human body including bearing loads in articular joints and intervertebral discs, providing joint lubrication, forming the external ears and nose, supporting the trachea, and forming the long bones during development and growth. The structure and organization of cartilage's extracellular matrix (ECM) are the primary determinants of normal function. Most diseases involving cartilage lead to dramatic changes in the ECM which can govern disease progression (e. Read More

To investigate the efficacy, recurrence and strategy of T silicone tube dilatation for cicatricial stenosis of cervical trachea in adults.Cinical data of 37 cases with the cervical tracheal stenosis were retrospectively analyzed, who were firstly treated by T shape silicone tube dilation for at least 10 months, from Jun 2006 to Jun 2016.In 37 adult patients with cicatricial stenosis of the trachea, 29 cases were caused by tracheal intubation or incision, 6 cases were traumatic, and 2 cases were multiple osteochondritis. Read More

Purpose: To investigate the clinical characteristics of Japanese patients with scleritis STUDY DESIGN: Retrospective study METHODS: The clinical records of 123 patients with scleritis, who presented to the University of Tokyo Hospital between January, 2004 and December, 2015, were retrospectively reviewed.

Background: Podocyte phospholipase A receptor (PLAR) is a major target antigen in idiopathic adult membranous nephropathy (MN). Histological PLAR staining in the renal tissue has proven to be useful for the detection of idiopathic MN. However, glomerular PLAR deposits have also been recently observed in several patients with secondary MN, such as hepatitis B virus-associated, hepatitis C virus-associated, and neoplasm-associated MN. Read More

Background: Relapsing polychondritis (RP) is a rare autoimmune disorder, and myelodysplastic syndrome (MDS) is accompanied by RP at variable rates. Herein, we report a case with RP and MDS who responded dramatically to 5-azacitidine for MDS.

Case Presentation: With conventional immunosuppressive treatment, our patient had several episodes of different side effects, including infections. Read More

Background: Relapsing polychondritis (RP) is an uncommon immune-related disease with unknown causes. It is characterized by inflammation of cartilaginous or non-cartilaginous structures, such as the ears, nose, respiratory tract, eyes, and joints. Neurological involvement is rare in RP. Read More

Authors:

Rare autoimmune diseases are difficult to conduct researches in spite of present era with advanced scientific progress. Research using genetic approach is a promising way since genetic findings implicate causality of diseases. Still, there are multiple obstacles preventing genetic studies of rare diseases. Read More

Objectives: To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP).

Methods: We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months. Read More

Authors:

Clinic of Otorhinolaryngology, Gülhane Training and Research Hospital, Ankara, Turkey.

Relapsing polychondritis (RP) is a rare autoimmune and inflammatory disease, particularly characterized by recurrent inflammation of the hyaline cartilage. Laryngotracheal involvement in RP is the most serious complication that is observed in 50% of the patients and may lead to a life-threatening condition. The most common cause of death is laryngotracheal stenosis associated with lung infections or severe respiratory insufficiency that may be observed in 10%-50% of the patients. Read More

Authors:

Relapsing polychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence of this rare disease are unknown. Read More

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